Status epilepticus: Case

This boy is in established convulsive status epilepticus bordering on refractory. The seizure has lasted twenty-five minutes, which places him just short of the ILAE t2 of thirty minutes, and he has already received two benzodiazepine doses without effect. His glucose is normal, so hypoglycaemia is excluded, and his fever and his first-presentation status raise a central nervous system infection as the likely trigger. The priority is to give a second-line agent at once and to prepare for refractory escalation. [1]

Clinical findings and assessment

The key findings are the continuous convulsion at twenty-five minutes, the failure of two adequate benzodiazepine doses, the fever of 39.0 degrees, and the normal glucose. The boy has crossed the t1 threshold of five minutes and is approaching the t2 threshold of thirty minutes, which places him in the established phase bordering on refractory. Because two benzodiazepine doses have failed, the next step is a second-line agent, not a third benzodiazepine, since the GABA-A receptors have internalised and further benzodiazepine doses add respiratory depression without adding efficacy. [2]

The differential of a first febrile presentation in status includes a central nervous system infection such as meningitis or encephalitis, a complex febrile seizure that has evolved into febrile status, and a first presentation of epilepsy provoked by fever. The absence of a prior seizure history and the high fever make an infectious cause the working diagnosis, which directs the investigation toward the blood culture, the lumbar puncture once stable, and the empirical antibiotics and aciclovir. [5]

Immediate management

The immediate management is to give a second-line agent now. My preferred agent is levetiracetam at 40 mg per kg intravenously, which is 760 mg for a 19 kg child, to a maximum of 4.5 g, infused over five minutes. The alternative is fosphenytoin at 20 mg phenytoin-equivalent per kg, which is 380 mg phenytoin-equivalent, to a maximum of 1500 mg phenytoin-equivalent, over ten minutes with cardiac monitoring. I would choose levetiracetam because the ConSEPT and EcLiPSE trials showed it is at least as effective as phenytoin in children with a better safety profile, and ESETT confirmed that levetiracetam, fosphenytoin, and valproate are equally effective across the age range. [3][4]

If the seizure does not stop with the second-line agent, the boy will have refractory status epilepticus, and I would escalate to rapid sequence intubation to protect the airway and ventilate, followed by a continuous midazolam, propofol, or thiopentone infusion titrated to burst suppression on the EEG. Continuous EEG monitoring would be mandatory from the moment of intubation, because the boy would be paralysed and the cortical seizure could continue unseen, and the only way to know whether the treatment is working is the EEG. [2]

Cause search and intensive care

The reversible causes are sought and treated in parallel with the drugs. The fever and the first-presentation status make a central nervous system infection the priority, so I would send a full blood count, a blood culture, electrolytes including calcium and magnesium, a venous gas, and a C-reactive protein. Once the seizure is controlled and the coagulation and platelets are checked, I would perform a lumbar puncture and start empirical antibiotics and aciclovir without delay. A computed tomography would be performed once the boy is stable, and a magnetic resonance imaging later to seek a structural cause if the workup is otherwise negative. [5]

The boy would be admitted to the paediatric intensive care unit, because he has required a second-line agent and is at high risk of refractory progression and of non-convulsive status once the convulsion is controlled. The intensive care team would manage the airway, the ventilation, and the anaesthetic infusion, with continuous EEG to confirm that the brain is electrically controlled. The paediatric neurology and infectious disease teams would be involved early to guide the cause search and the longer-term plan. [5]

Outcome and follow-up

The prognosis of a first febrile presentation in status depends on the underlying cause and on the duration of the seizure before control. The fever and the infectious cause are reassuring in the sense that they are treatable, and the boy has a good chance of full recovery if the seizure is controlled promptly and the infection is treated. The risk of subsequent epilepsy is elevated after a first presentation in status, and the risk of recurrence of status is about one in six, which is why the boy would be followed up in a paediatric neurology clinic. [5]

I would counsel the family honestly at every stage. I would explain that their son has been in a prolonged seizure that did not stop on its own, that the medicines are being given in a stepwise order to stop it, and that the blood tests and the lumbar puncture are looking for an infection that can be treated. I would explain that if the seizure does not stop with the second drug he will need a breathing machine and intensive care, that his brain will be watched with an EEG throughout, and that the team will return to explain the findings and the plan as soon as the seizure is controlled. Before discharge, the family would be connected to the paediatric neurology service, and a rescue plan would be discussed for any future episode. [5]